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Abnormal autophagy, ubiquitination, inflammation and apoptosis are dependent upon lysosomal storage and are useful biomarkers of mucopolysaccharidosis VI

Alessandra Tessitore1, Marinella Pirozzi1 and Alberto Auricchio1,2

Telethon Institute of Genetics and Medicine (TIGEM), Naples, Italy

Medical Genetics, Department of Pediatrics, 'Federico II' University, Naples, Italy

PathoGenetics 2009, 2:4doi:10.1186/1755-8417-2-4

Published: 16 June 2009

Additional files


Additional file 1:

Epidermal Growth Factor (EGF)/EGF Receptor (EGFR) turnover in NR fibroblasts. Normal skin fibroblasts were loaded with LysoTracker (red) and EGF (green) to follow the EGF/EGFR turnover via lysosomal degradation. Image sequences were recorded at one frame per 15 min, played at 3 frames/sec, and cover 20 sec (5 h). Time is indicated in the upper left corner and is hours:minutes:seconds. Scale bar = 20 μm.

Format: MOV Size: 9MB Download file

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Open Data


Additional file 2:

Epidermal Growth Factor (EGF)/EGF Receptor (EGFR) turnover in MPS VI fibroblasts. MPS VI skin fibroblasts were loaded with LysoTracker (red) and EGF (green) to follow the EGF/EGFR turnover via lysosomal degradation. Image sequences were recorded at one frame per 15 min, played at 3 frames/sec, and cover 20 sec (9 h). Time is indicated in the upper left corner and is hours:minutes:seconds. Scale bar = 20 μm.

Format: MOV Size: 4.2MB Download file

Playing the movie within this page requires QuickTime and JavaScript. Read more

Open Data

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